{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T10:04:49Z","timestamp":1790071489565,"version":"4.0.1"},"reference-count":135,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2008,3,1]],"date-time":"2008-03-01T00:00:00Z","timestamp":1204329600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Research Reviews"],"published-print":{"date-parts":[[2008,3]]},"DOI":"10.1016\/j.brainresrev.2007.05.012","type":"journal-article","created":{"date-parts":[[2007,6,14]],"date-time":"2007-06-14T05:05:04Z","timestamp":1181797504000},"page":"410-420","source":"Crossref","is-referenced-by-count":73,"title":["Endogenous and synthetic neurosteroids in treatment of Niemann\u2013Pick Type C disease"],"prefix":"10.1016","volume":"57","author":[{"given":"Synthia H.","family":"Mellon","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenhui","family":"Gong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcus D.","family":"Schonemann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.brainresrev.2007.05.012_bib1","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1002\/jnr.20685","article-title":"Allopregnanolone treatment, both as a single injection or repetitively, delays demyelination and enhances survival of Niemann\u2013Pick C mice","volume":"82","author":"Ahmad","year":"2005","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib2","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/j.bbamem.2006.07.011","article-title":"Lack of enantiomeric specificity in the effects of anesthetic steroids on lipid bilayers","volume":"1768","author":"Alakoskela","year":"2007","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib3","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1055\/s-0028-1091741","article-title":"Niemann\u2013Pick disease type C: case report with ultrastructural findings","volume":"4","author":"Anzil","year":"1973","journal-title":"Neuropadiatrie"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib4","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1196\/annals.1286.005","article-title":"Pathogenesis in menstrual cycle-linked CNS disorders","volume":"1007","author":"Backstrom","year":"2003","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib5","doi-asserted-by":"crossref","first-page":"5383","DOI":"10.1128\/MCB.24.12.5383-5390.2004","article-title":"Deletion of the mouse P450c17 gene causes early embryonic lethality","volume":"24","author":"Bair","year":"2004","journal-title":"Mol. Cell Biol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib6","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1615\/CritRevNeurobiol.v16.i12.70","article-title":"Neurosteroidogenesis: relevance to neurosteroid actions in brain and modulation by psychotropic drugs","volume":"16","author":"Barbaccia","year":"2004","journal-title":"Crit. Rev. Neurobiol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib7","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1089\/neu.1995.12.1","article-title":"A sensitive and reliable locomotor rating scale for open field testing in rats","volume":"12","author":"Basso","year":"1995","journal-title":"J. Neurotrauma"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib8","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1002\/humu.10016","article-title":"NPC1: complete genomic sequence, mutation analysis, and characterization of haplotypes","volume":"19","author":"Bauer","year":"2002","journal-title":"Human Mutat."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib9","series-title":"Neurosteroids: A New Regulatory Function in the Nervous System","year":"1999"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib10","doi-asserted-by":"crossref","first-page":"868","DOI":"10.1016\/S0022-3565(24)37308-2","article-title":"Reversal of behavioral effects of pentylenetetrazol by the neuroactive steroid ganaxolone","volume":"284","author":"Beekman","year":"1998","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib11","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1016\/j.neuroscience.2005.07.021","article-title":"Neuroactive steroids and inhibitory neurotransmission: mechanisms of action and physiological relevance","volume":"138","author":"Belelli","year":"2006","journal-title":"Neuroscience"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib12","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1038\/nrn1703","article-title":"Neurosteroids: endogenous regulators of the GABA(A) receptor","volume":"6","author":"Belelli","year":"2005","journal-title":"Nat. Rev., Neurosci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib13","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1080\/09513590400018223","article-title":"Disadaptive disorders in women: allopregnanolone, a sensitive steroid","volume":"19","author":"Bernardi","year":"2004","journal-title":"Gynecol. Endocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib14","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1055\/s-2007-978871","article-title":"Allopregnanolone in women with premenstrual syndrome","volume":"30","author":"Bicikova","year":"1998","journal-title":"Horm. Metab. Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib15","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/S0006-8993(97)00455-1","article-title":"Progesterone, 5alpha-pregnane-3,20-dione and 3alpha-hydroxy-5alpha-pregnane-20-one in specific regions of the human female brain in different endocrine states","volume":"764","author":"Bixo","year":"1997","journal-title":"Brain Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib16","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1097\/00005072-198311000-00007","article-title":"Isocortical pathology in type C Niemann\u2013Pick disease. A combined Golgi\u2013pigmentoarchitectonic study","volume":"42","author":"Braak","year":"1983","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib17","doi-asserted-by":"crossref","first-page":"185","DOI":"10.2174\/156720506777632817","article-title":"Therapeutic potential of neurogenesis for prevention and recovery from Alzheimer's disease: allopregnanolone as a proof of concept neurogenic agent","volume":"3","author":"Brinton","year":"2006","journal-title":"Curr. Alzheimer. Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib18","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0014-2999(97)00096-4","article-title":"The anxiolytic-like effects of the neurosteroid allopregnanolone: interactions with GABA(A) receptors","volume":"325","author":"Brot","year":"1997","journal-title":"Eur. J. Pharmacol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib19","doi-asserted-by":"crossref","first-page":"1530","DOI":"10.1111\/j.1471-4159.2004.02686.x","article-title":"Fetal alcohol exposure alters neurosteroid levels in the developing rat brain","volume":"90","author":"Caldeira","year":"2004","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib20","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1126\/science.277.5323.228","article-title":"Niemann\u2013Pick C1 disease gene: homology to mediators of cholesterol homeostasis","volume":"277","author":"Carstea","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib21","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1016\/S0022-3565(24)36511-5","article-title":"Characterization of the anticonvulsant properties of ganaxolone (CCD 1042; 3alpha-hydroxy-3beta-methyl-5alpha-pregnan-20-one), a selective, high-affinity, steroid modulator of the gamma-aminobutyric acid(A) receptor","volume":"280","author":"Carter","year":"1997","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib22","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1002\/jbm.b.30243","article-title":"Ultrastructural analysis of guided nerve regeneration using progesterone- and pregnenolone-loaded chitosan prostheses","volume":"74","author":"Chavez-Delgado","year":"2005","journal-title":"J. Biomed. Mater. Res., Part B Appl. Biomater."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib23","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1006\/frne.1999.0188","article-title":"Neurosteroids: biosynthesis and function of these novel neuromodulators","volume":"21","author":"Compagnone","year":"2000","journal-title":"Front. Neuroendocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib24","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1016\/S0022-3565(24)39327-9","article-title":"Enantioselectivity of pregnanolone-induced gamma-aminobutyric acid(A) receptor modulation and anesthesia","volume":"293","author":"Covey","year":"2000","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib25","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1111\/j.1471-4159.1961.tb13499.x","article-title":"The cerebral defect in Tay\u2013Sachs disease and Niemann\u2013Pick disease","volume":"7","author":"Crocker","year":"1961","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib26","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/j.expneurol.2005.06.003","article-title":"Tapered progesterone withdrawal enhances behavioral and molecular recovery after traumatic brain injury","volume":"195","author":"Cutler","year":"2005","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib27","doi-asserted-by":"crossref","first-page":"2295","DOI":"10.1126\/science.290.5500.2295","article-title":"Transmembrane molecular pump activity of Niemann\u2013Pick C1 protein","volume":"290","author":"Davies","year":"2000","journal-title":"Science"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib28","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S0304-3940(00)00965-4","article-title":"Increased neurosteroids synthesis after brain and spinal cord injury in rats","volume":"284","author":"di Michele","year":"2000","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib29","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1089\/neu.2005.22.106","article-title":"The neurosteroids progesterone and allopregnanolone reduce cell death, gliosis, and functional deficits after traumatic brain injury in rats","volume":"22","author":"Djebaili","year":"2005","journal-title":"J. Neurotrauma"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib30","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1001\/archpsyc.59.9.851","article-title":"Cortical gamma-aminobutyric acid levels across the menstrual cycle in healthy women and those with premenstrual dysphoric disorder: a proton magnetic resonance spectroscopy study","volume":"59","author":"Epperson","year":"2002","journal-title":"Arch. Gen. Psychiatry"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib31","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1212\/WNL.39.8.1040","article-title":"Clinical spectrum of Niemann\u2013Pick disease type C","volume":"39","author":"Fink","year":"1989","journal-title":"Neurology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib33","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1016\/j.pbb.2003.09.004","article-title":"Neurosteroid consumption has anxiolytic effects in mice","volume":"76","author":"Finn","year":"2003","journal-title":"Pharmacol. Biochem. Behav."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib32","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.pharmthera.2003.10.006","article-title":"The role of pregnane neurosteroids in ethanol withdrawal: behavioral genetic approaches","volume":"101","author":"Finn","year":"2004","journal-title":"Pharmacol. Ther."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib34","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1002\/jnr.10821","article-title":"Treatment with the neurosteroid dehydroepiandrosterone promotes recovery of motor behavior after moderate contusive spinal cord injury in the mouse","volume":"75","author":"Fiore","year":"2004","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib35","doi-asserted-by":"crossref","first-page":"1567","DOI":"10.1073\/pnas.89.5.1567","article-title":"Memory-enhancing effects in male mice of pregnenolone and steroids metabolically derived from it","volume":"89","author":"Flood","year":"1992","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib36","doi-asserted-by":"crossref","first-page":"10806","DOI":"10.1073\/pnas.92.23.10806","article-title":"Pregnenolone sulfate enhances post-training memory processes when injected in very low doses into limbic system structures: the amygdala is by far the most sensitive","volume":"92","author":"Flood","year":"1995","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib37","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.ejphar.2004.07.041","article-title":"Modulation of GABA(A) receptor gene expression by allopregnanolone and ethanol","volume":"500","author":"Follesa","year":"2004","journal-title":"Eur. J. Pharmacol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib38","first-page":"191","article-title":"Gamma-aminobutyric acidA receptor regulation: chronic treatment with pregnanolone uncouples allosteric interactions between steroid and benzodiazepine recognition sites","volume":"44","author":"Friedman","year":"1993","journal-title":"Mol. Pharmacol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib39","doi-asserted-by":"crossref","first-page":"25517","DOI":"10.1074\/jbc.M302588200","article-title":"NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols","volume":"278","author":"Frolov","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib40","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/glia.1117","article-title":"Progesterone and the oligodendroglial lineage: stage-dependent biosynthesis and metabolism","volume":"36","author":"Gago","year":"2001","journal-title":"Glia"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib41","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1016\/S0028-3908(99)00190-2","article-title":"Acute and chronic effects of the synthetic neuroactive steroid, ganaxolone, against the convulsive and lethal effects of pentylenetetrazol in seizure-kindled mice: comparison with diazepam and valproate","volume":"39","author":"Gasior","year":"2000","journal-title":"Neuropharmacology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib42","first-page":"55","article-title":"Epalons as anticonvulsants: actions mediated by the GABAA receptor complex","volume":"39","author":"Gee","year":"1996","journal-title":"Proc. West Pharmacol. Soc."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib43","doi-asserted-by":"crossref","first-page":"1778","DOI":"10.1210\/me.2003-0323","article-title":"Aberrant intracellular cholesterol transport disrupts pituitary and ovarian function","volume":"18","author":"Gevry","year":"2004","journal-title":"Mol. Endocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib44","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1046\/j.1471-4159.2003.01881.x","article-title":"Progesterone and its metabolites increase myelin basic protein expression in organotypic slice cultures of rat cerebellum","volume":"86","author":"Ghoumari","year":"2003","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib45","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.neuroscience.2005.05.023","article-title":"Progesterone increases oligodendroglial cell proliferation in rat cerebellar slice cultures","volume":"135","author":"Ghoumari","year":"2005","journal-title":"Neuroscience"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib46","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1007\/BF00442993","article-title":"Niemann\u2013Pick disease type C. Pathological, histochemical, ultrastructural and biochemical studies","volume":"136","author":"Gilbert","year":"1981","journal-title":"Eur. J. Pediatr."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib47","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1016\/S0006-3223(00)01044-1","article-title":"Allopregnanolone levels and reactivity to mental stress in premenstrual dysphoric disorder","volume":"49","author":"Girdler","year":"2001","journal-title":"Biol. Psychiatry"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib48","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1038\/nm1073","article-title":"Niemann\u2013Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone","volume":"10","author":"Griffin","year":"2004","journal-title":"Nat. Med."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib49","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1007\/s00213-004-2084-3","article-title":"Cortical 3 alpha-hydroxy-5 alpha-pregnan-20-one levels after acute administration of Delta 9-tetrahydrocannabinol, cocaine and morphine","volume":"179","author":"Grobin","year":"2005","journal-title":"Psychopharmacology (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib50","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1196\/annals.1286.012","article-title":"Neurosteroids in the retina: neurodegenerative and neuroprotective agents in retinal degeneration","volume":"1007","author":"Guarneri","year":"2003","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib51","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/0006-8993(84)90299-3","article-title":"Modulation of GABA receptor complex by a steroid anesthetic","volume":"323","author":"Harrison","year":"1984","journal-title":"Brain Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib52","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1007\/BF00227765","article-title":"Cerebellar degeneration in the Niemann\u2013Pick type C mouse","volume":"85","author":"Higashi","year":"1993","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib53","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1007\/BF00294315","article-title":"Peripheral nerve pathology in Niemann\u2013Pick type C mouse","volume":"90","author":"Higashi","year":"1995","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib54","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1089\/089771503322385791","article-title":"The delayed administration of dehydroepiandrosterone sulfate improves recovery of function after traumatic brain injury in rats","volume":"20","author":"Hoffman","year":"2003","journal-title":"J. Neurotrauma"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib55","doi-asserted-by":"crossref","first-page":"1943","DOI":"10.1210\/me.2002-0055","article-title":"Steroid deficiency syndromes in mice with targeted disruption of Cyp11a1","volume":"16","author":"Hu","year":"2002","journal-title":"Mol. Endocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib56","first-page":"2055","article-title":"Effects of chronic ethanol consumption and withdrawal on the neuroactive steroid 3alpha-hydroxy-5alpha-pregnan-20-one in male and female rats","volume":"22","author":"Janis","year":"1998","journal-title":"Alcohol. Clin. Exp. Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib57","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.devbrainres.2004.07.009","article-title":"Role of allopregnanolone on cerebellar granule cells neurogenesis","volume":"153","author":"Keller","year":"2004","journal-title":"Brain Res. Dev. Brain Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib58","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/S0022-3476(05)81695-6","article-title":"Niemann\u2013Pick disease type C: diagnosis and outcome in children, with particular reference to liver disease","volume":"123","author":"Kelly","year":"1993","journal-title":"J. Pediatr."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib59","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/S0920-1211(00)00170-4","article-title":"Ganaxolone for treating intractable infantile spasms: a multicenter, open-label, add-on trial","volume":"42","author":"Kerrigan","year":"2000","journal-title":"Epilepsy Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib60","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1210\/er.2001-0038","article-title":"The nuclear pregnane X receptor: a key regulator of xenobiotic metabolism","volume":"23","author":"Kliewer","year":"2002","journal-title":"Endocr. Rev."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib61","doi-asserted-by":"crossref","first-page":"1847","DOI":"10.1210\/en.2005-0955","article-title":"Injury elicited increase in spinal cord neurosteroid content analyzed by gas chromatography mass spectrometry","volume":"147","author":"Labombarda","year":"2006","journal-title":"Endocrinology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib62","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/j.taap.2003.12.027","article-title":"PXR (NR1I2): splice variants in human tissues, including brain, and identification of neurosteroids and nicotine as PXR activators","volume":"199","author":"Lamba","year":"2004","journal-title":"Toxicol. Appl. Pharmacol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib63","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.pneurobio.2003.09.001","article-title":"Neurosteroid modulation of GABA(A) receptors","volume":"71","author":"Lambert","year":"2003","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib64","doi-asserted-by":"crossref","first-page":"13807","DOI":"10.1073\/pnas.0606218103","article-title":"Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann\u2013Pick C disease","volume":"103","author":"Langmade","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib65","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1016\/j.brainres.2003.10.047","article-title":"The neuroactive steroid 3-alpha-ol-5-beta-pregnan-20-one hemisuccinate, a selective NMDA receptor antagonist improves behavioral performance following spinal cord ischemia","volume":"997","author":"Lapchak","year":"2004","journal-title":"Brain Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib66","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1111\/j.1528-1157.2000.tb00324.x","article-title":"Assessment of ganaxolone's anticonvulsant activity using a randomized, double-blind, presurgical trial design. Ganaxolone Presurgical Study Group","volume":"41","author":"Laxer","year":"2000","journal-title":"Epilepsia"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib67","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1002\/(SICI)1098-1136(20000101)29:1<14::AID-GLIA2>3.0.CO;2-N","article-title":"Neurosteroid progesterone is up-regulated in the brain of jimpy and shiverer mice","volume":"29","author":"Le Goascogne","year":"2000","journal-title":"Glia"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib68","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1126\/science.277.5323.232","article-title":"Murine model of Niemann\u2013Pick C disease: mutation in a cholesterol homeostasis gene","volume":"277","author":"Loftus","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib70","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1210\/me.11.7.917","article-title":"Fetal death in mice lacking 5alpha-reductase type 1 caused by estrogen excess","volume":"11","author":"Mahendroo","year":"1997","journal-title":"Mol. Endocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib71","doi-asserted-by":"crossref","first-page":"4652","DOI":"10.1210\/en.142.11.4652","article-title":"Unexpected virilization in male mice lacking steroid 5 alpha-reductase enzymes","volume":"142","author":"Mahendroo","year":"2001","journal-title":"Endocrinology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib69","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.mce.2004.04.009","article-title":"Steroid 5alpha-reductase 1 promotes 5alpha-androstane-3alpha,17beta-diol synthesis in immature mouse testes by two pathways","volume":"222","author":"Mahendroo","year":"2004","journal-title":"Mol. Cell. Endocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib72","doi-asserted-by":"crossref","first-page":"1004","DOI":"10.1126\/science.2422758","article-title":"Steroid hormone metabolites are barbiturate-like modulators of the GABA receptor","volume":"232","author":"Majewska","year":"1986","journal-title":"Science"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib73","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1007\/BF02245063","article-title":"The neurosteroid pregnenolone sulfate blocks NMDA antagonist-induced deficits in a passive avoidance memory task","volume":"116","author":"Mathis","year":"1994","journal-title":"Psychopharmacology (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib74","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1016\/0006-8993(93)91524-V","article-title":"Infusion of neurosteroids into the nucleus basalis magnocellularis affects cognitive processes in the rat","volume":"607","author":"Mayo","year":"1993","journal-title":"Brain Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib75","doi-asserted-by":"crossref","first-page":"2505","DOI":"10.1210\/en.2006-1678","article-title":"Steroid profiling in brain and plasma of male and pseudopregnant female rats after traumatic brain injury: analysis by gas chromatography\/mass spectrometry","volume":"148","author":"Meffre","year":"2007","journal-title":"Endocrinology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib76","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/S0165-0173(01)00110-2","article-title":"Anatomical and biochemical evidence for the synthesis of unconjugated and sulfated neurosteroids in amphibians","volume":"37","author":"Mensah-Nyagan","year":"2001","journal-title":"Brain Res. Brain Res. Rev."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib77","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1086\/324068","article-title":"Niemann\u2013Pick disease type C: spectrum of HE1 mutations and genotype\/phenotype correlations in the NPC2 group","volume":"69","author":"Millat","year":"2001","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib78","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1210\/edrv-9-3-295","article-title":"Molecular biology of steroid hormone synthesis","volume":"9","author":"Miller","year":"1988","journal-title":"Endocr. Rev."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib79","series-title":"Endocrinology and Metabolism","first-page":"555","article-title":"The adrenal cortex","author":"Miller","year":"1994"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib80","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1111\/j.1528-1157.1997.tb01486.x","article-title":"Initial human experience with ganaxolone, a neuroactive steroid with antiepileptic activity","volume":"38","author":"Monaghan","year":"1997","journal-title":"Epilepsia"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib81","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1530\/eje.0.1420269","article-title":"Allopregnanolone concentrations and premenstrual syndrome","volume":"142","author":"Monteleone","year":"2000","journal-title":"Eur. J. Endocrinol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib82","doi-asserted-by":"crossref","first-page":"15122","DOI":"10.1074\/jbc.M001215200","article-title":"Orphan nuclear receptors constitutive androstane receptor and pregnane X receptor share xenobiotic and steroid ligands","volume":"275","author":"Moore","year":"2000","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib83","first-page":"140","article-title":"Lysosome lipid storage disorder in NCTR-BALB\/c mice: I. Description of the disease and genetics","volume":"108","author":"Morris","year":"1982","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib84","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/S0074-7742(01)46068-5","article-title":"Ethanol and neurosteroid interactions in the brain","volume":"46","author":"Morrow","year":"2001","journal-title":"Int. Rev. Neurobiol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib85","doi-asserted-by":"crossref","first-page":"2298","DOI":"10.1126\/science.290.5500.2298","article-title":"Identification of HE1 as the second gene of Niemann\u2013Pick C disease","volume":"290","author":"Naureckiene","year":"2000","journal-title":"Science"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib86","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1136\/adc.42.221.91","article-title":"The juvenile form of Niemann\u2013Pick disease","volume":"42","author":"Norman","year":"1967","journal-title":"Arch. Dis. Child"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib87","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1016\/j.pain.2005.01.009","article-title":"New evidence that both T-type calcium channels and GABAA channels are responsible for the potent peripheral analgesic effects of 5alpha-reduced neuroactive steroids","volume":"114","author":"Pathirathna","year":"2005","journal-title":"Pain"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib88","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1002\/cne.20251","article-title":"Anatomical and cellular localization of neuroactive 5 alpha\/3 alpha-reduced steroid-synthesizing enzymes in the spinal cord","volume":"477","author":"Patte-Mensah","year":"2004","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib89","first-page":"3611","article-title":"Niemann\u2013Pick disease type C: a lipid trafficking disorder","volume":"Vol. III","author":"Patterson","year":"2001"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib90","first-page":"2625","article-title":"Niemann\u2013Pick disease type C: a cellular cholesterol lipidosis","author":"Pentchev","year":"1995"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib91","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S0165-3806(99)00181-9","article-title":"In vivo evidences of early neurosteroid synthesis in the developing rat central nervous system and placenta","volume":"120","author":"Pomata","year":"2000","journal-title":"Brain Res. Dev. Brain Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib92","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1016\/S0029-7844(97)00417-1","article-title":"Progesterone metabolite allopregnanolone in women with premenstrual syndrome","volume":"90","author":"Rapkin","year":"1997","journal-title":"Obstet. Gynecol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib93","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1358\/dot.2002.38.7.820115","article-title":"The clinical potentials of endogenous neurosteroids","volume":"38","author":"Reddy","year":"2002","journal-title":"Drugs Today (Barc.)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib94","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.eplepsyres.2004.09.003","article-title":"Role of neurosteroids in catamenial epilepsy","volume":"62","author":"Reddy","year":"2004","journal-title":"Epilepsy Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib95","first-page":"1241","article-title":"Chronic treatment with the neuroactive steroid ganaxolone in the rat induces anticonvulsant tolerance to diazepam but not to itself","volume":"295","author":"Reddy","year":"2000","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib96","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1016\/S0022-3565(24)39152-9","article-title":"Enhanced anticonvulsant activity of ganaxolone after neurosteroid withdrawal in a rat model of catamenial epilepsy","volume":"294","author":"Reddy","year":"2000","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib97","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/0960-0760(95)00074-A","article-title":"Biosynthesis and assay of neurosteroids in rats and mice: functional correlates","volume":"53","author":"Robel","year":"1995","journal-title":"J. Steroid. Biochem. Mol. Biol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib98","first-page":"1","article-title":"Allopregnanolone and ganaxolone increase the firing activity of dorsal raphe nucleus serotonergic neurons in female rats","author":"Robichaud","year":"2005","journal-title":"Int. J."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib99","doi-asserted-by":"crossref","first-page":"2913","DOI":"10.1210\/en.133.6.2913","article-title":"The murine Niemann\u2013Pick type C lesion affects testosterone production","volume":"133","author":"Roff","year":"1993","journal-title":"Endocrinology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib100","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/S0074-7742(02)49014-9","article-title":"Neurosteroids and infantile spasms: the deoxycorticosterone hypothesis","volume":"49","author":"Rogawski","year":"2002","journal-title":"Int. Rev. Neurobiol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib101","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S0039-128X(98)00101-9","article-title":"Neuropsychopharmacological properties of neuroactive steroids","volume":"64","author":"Rupprecht","year":"1999","journal-title":"Steroids"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib102","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1210\/jc.79.5.1256","article-title":"Circulating levels of anxiolytic steroids in the luteal phase in women with premenstrual syndrome and in control subjects","volume":"79","author":"Schmidt","year":"1994","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib103","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1023\/A:1007152904926","article-title":"Steroid synthesis and metabolism in the nervous system: trophic and protective effects","volume":"29","author":"Schumacher","year":"2000","journal-title":"J. Neurocytol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib104","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.pneurobio.2003.09.004","article-title":"Steroid hormones and neurosteroids in normal and pathological aging of the nervous system","volume":"71","author":"Schumacher","year":"2003","journal-title":"Prog. Neurobiol."},{"issue":"Suppl A","key":"10.1016\/j.brainresrev.2007.05.012_bib105","doi-asserted-by":"crossref","first-page":"S18","DOI":"10.1016\/j.ghir.2004.03.007","article-title":"Local synthesis and dual actions of progesterone in the nervous system: neuroprotection and myelination","volume":"14","author":"Schumacher","year":"2004","journal-title":"Growth Horm. IGF Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib106","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1006\/exnr.2002.8020","article-title":"Progesterone protects against necrotic damage and behavioral abnormalities caused by traumatic brain injury","volume":"178","author":"Shear","year":"2002","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib107","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1038\/nn1868","article-title":"Reversal of neurosteroid effects at alpha4beta2delta GABA(A) receptors triggers anxiety at puberty","volume":"10","author":"Shen","year":"2007","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib108","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1038\/31948","article-title":"GABAA receptor \u03b14 subunit suppression prevents withdrawal properties of an endogenous steroid","volume":"392","author":"Smith","year":"1998","journal-title":"Nature"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib109","series-title":"The Metabolic Basis of Inherited Disease","first-page":"1655","article-title":"Sphingomyelin\u2013cholesterol lipidoses: the Niemann\u2013Pick group of diseases","author":"Spence","year":"1989"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib110","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1196\/annals.1286.007","article-title":"Neurosteroid biosynthesis in the human brain and its clinical implications","volume":"1007","author":"Stoffel-Wagner","year":"2003","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib111","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1007\/BF00309338","article-title":"Neurofibrillary tangles in Niemann\u2013Pick disease type C","volume":"89","author":"Suzuki","year":"1995","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib112","doi-asserted-by":"crossref","first-page":"3202","DOI":"10.1073\/pnas.0307325101","article-title":"Mitotic and neurogenic effects of dehydroepiandrosterone (DHEA) on human neural stem cell cultures derived from the fetal cortex","volume":"101","author":"Suzuki","year":"2004","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib113","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1007\/s00213-005-0148-7","article-title":"Pharmacokinetic and behavioral effects of allopregnanolone in healthy women","volume":"186","author":"Timby","year":"2006","journal-title":"Psychopharmacology (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib114","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1124\/mol.104.002402","article-title":"5beta-reduced neuroactive steroids are novel voltage-dependent blockers of T-type Ca2+ channels in rat sensory neurons in vitro and potent peripheral analgesics in vivo","volume":"66","author":"Todorovic","year":"2004","journal-title":"Mol. Pharmacol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib115","doi-asserted-by":"crossref","first-page":"73","DOI":"10.2174\/187152406776056555","article-title":"Neurosteroids in the brain neuron: biosynthesis, action and medicinal impact on neurodegenerative disease","volume":"6","author":"Tsutsui","year":"2006","journal-title":"Cent. Nerv. Syst. Agents Med. Chem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib116","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.neures.2004.03.006","article-title":"Organizing actions of neurosteroids in the Purkinje neuron","volume":"49","author":"Tsutsui","year":"2004","journal-title":"Neurosci. Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib117","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1007\/s002130050060","article-title":"Modification of behavioral effects of drugs in mice by neuroactive steroids","volume":"148","author":"Ungard","year":"2000","journal-title":"Psychopharmacology (Berl)"},{"issue":"Berl","key":"10.1016\/j.brainresrev.2007.05.012_bib118","first-page":"1","article-title":"Relevance of endogenous 3alpha-reduced neurosteroids to depression and antidepressant action","author":"Uzunova","year":"2005","journal-title":"Psychopharmacology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib119","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/S0074-7742(01)46066-1","article-title":"Neurosteroids in learning and memory processes","volume":"46","author":"Vallee","year":"2001","journal-title":"Int. Rev. Neurobiol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib120","doi-asserted-by":"crossref","first-page":"1982","DOI":"10.1523\/JNEUROSCI.20-05-01982.2000","article-title":"Neuroactive steroid 3alpha-hydroxy-5alpha-pregnan-20-one modulates electrophysiological and behavioral actions of ethanol","volume":"20","author":"VanDoren","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib121","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1111\/j.1399-0004.1988.tb03460.x","article-title":"Niemann\u2013Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients","volume":"33","author":"Vanier","year":"1988","journal-title":"Clin. Genet."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib122","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1007\/BF01797928","article-title":"Niemann\u2013Pick disease type C: an update","volume":"14","author":"Vanier","year":"1991","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib123","first-page":"118","article-title":"Genetic heterogeneity in Niemann\u2013Pick C disease: a study using somatic cell hybridization and linkage analysis","volume":"58","author":"Vanier","year":"1996","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib124","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1016\/j.neuropharm.2006.07.015","article-title":"The enantiomer of progesterone acts as a molecular neuroprotectant after traumatic brain injury","volume":"51","author":"VanLandingham","year":"2006","journal-title":"Neuropharmacology"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib125","doi-asserted-by":"crossref","first-page":"4706","DOI":"10.1523\/JNEUROSCI.4520-04.2005","article-title":"The neurosteroid allopregnanolone promotes proliferation of rodent and human neural progenitor cells and regulates cell-cycle gene and protein expression","volume":"25","author":"Wang","year":"2005","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib126","doi-asserted-by":"crossref","first-page":"2329","DOI":"10.1126\/science.1060762","article-title":"The human nuclear xenobiotic receptor PXR: structural determinants of directed promiscuity","volume":"292","author":"Watkins","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib127","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1111\/j.1471-4159.1985.tb04044.x","article-title":"Neurological mutation characterized by dysmyelination in NCTR\u2013Balb\/C mouse with lysosomal lipid storage disease","volume":"45","author":"Weintraub","year":"1985","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib128","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1007\/BF00687215","article-title":"Dysmyelination in NCTR\u2013Balb\/C mouse mutant with a lysosomal storage disorder. Morphological survey","volume":"74","author":"Weintraub","year":"1987","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib129","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1001\/archpedi.1977.02120220021002","article-title":"Nine cases of sphingomyelin lipidosis, a new variant in Spanish-American Children. Juvenile variant of Niemann\u2013Pick Disease with foamy and sea-blue histiocytes","volume":"131","author":"Wenger","year":"1977","journal-title":"Am. J. Dis. Child."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib130","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1002\/dvg.20266","article-title":"Human CYP11A1 promoter drives Cre recombinase expression in the brain in addition to adrenals and gonads","volume":"45","author":"Wu","year":"2007","journal-title":"Genesis"},{"key":"10.1016\/j.brainresrev.2007.05.012_bib131","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1093\/jnen\/59.12.1106","article-title":"Cholesterol is sequestered in the brains of mice with Niemann\u2013Pick type C disease but turnover is increased","volume":"59","author":"Xie","year":"2000","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib132","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1093\/jnen\/60.1.49","article-title":"Neurons in Niemann\u2013Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations","volume":"60","author":"Zervas","year":"2001","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib133","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1194\/jlr.M300409-JLR200","article-title":"The steroidal analog GW707 activates the SREBP pathway through disruption of intracellular cholesterol trafficking","volume":"45","author":"Zhang","year":"2004","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib134","doi-asserted-by":"crossref","first-page":"4022","DOI":"10.1523\/JNEUROSCI.17-11-04022.1997","article-title":"Neurosteroid prolongs GABAA channel deactivation by altering kinetics of desensitized states","volume":"17","author":"Zhu","year":"1997","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresrev.2007.05.012_bib135","doi-asserted-by":"crossref","first-page":"6648","DOI":"10.1523\/JNEUROSCI.16-21-06648.1996","article-title":"Delta subunit inhibits neurosteroid modulation of GABAA receptors","volume":"16","author":"Zhu","year":"1996","journal-title":"J. Neurosci."}],"container-title":["Brain Research Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0165017307000975?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0165017307000975?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T06:59:55Z","timestamp":1737097195000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0165017307000975"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,3]]},"references-count":135,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2008,3]]}},"alternative-id":["S0165017307000975"],"URL":"https:\/\/doi.org\/10.1016\/j.brainresrev.2007.05.012","relation":{},"ISSN":["0165-0173"],"issn-type":[{"value":"0165-0173","type":"print"}],"subject":[],"published":{"date-parts":[[2008,3]]}}}